[Tetralogy of Fallot with congenital absence of a pulmonary branch. Apropos of 4 cases].
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Biomedical subjects
Publications and source records attributed to J Y Neveux.
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We present our experience in the management of tricuspid atresia in 115 children. The anatomic data are categorized as follows: type I, 83.5%, type II, 16.5%. Type IB is the most frequent, representing 63.5% of all the cases. Each patient was operated upon one to four times. The age at first operation ranged from 10 days to 20 years. The first operation was a shunt in 94 children, a Fontan operation in four, and banding of the pulmonary artery in 17. Hospital mortality for the first operation was 12.2%, significantly higher in children under 6 months and in those having Waterston shunts. Potts and Blalock-Taussig operations give low long-term mortality; although few (six) have been done, Potts shunts also seem to give good long-term palliation in this series. The Glenn anastomosis is a good operation when performed after a systemic-pulmonary arterial shunt. The Fontan operation was performed in 24 children (hospital mortality 16.6%). There have been no late deaths after the third month postoperatively. Mean follow-up for this operation is only 2 years, but 88% of the survivors lead a normal life, two thirds of them receiving no treatment. There has been one reoperation for stenosis of a Dacron conduit with a good result. Late arrhythmias are well tolerated. In conclusion, the Fontan procedure is a good operation, but palliative procedures still allow good long-term survival.
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Between 1972 and 1978, forty-six infants under six months of age underwent surgery for isolated coarctation of the aorta. These forty-six patients represent 32% of the total number of infants aged less than six months who had surgery for coarctation of the aorta during the same period. At the time of operation, 41% were aged less than one month and the youngest was five days old. The main cause for surgery was heart failure in infants under one month (14/19) and severe systemic hypertension (150 to 300 mm Hg) in the one to six month age group (18/27). Overall mortality rate in our series was 17%. Six of the eight infants who died were under one month of age. Recoarctation occurred in 31,5% of infants; in eight cases the first surgical procedure had been done before one month of age. A second procedure was necessary in four cases. Early surgical repair of severe coarctation diagnosed during the first six months of life leads to functional improvement and avoids residual hypertension. After repair, the main risk is recoarctation.
In spite of the availability of small size models, intra-abdominal implantation of epicardial pacemakers is difficult in infants. The alternative, which is implantation within the pleural cavity, entails a risk of compression of the lung. These problems can be overcome by using a totally extrapleural thoracic approach.
A series of 25 congenital malformations of the atrioventricular valves underwent valve replacement (10 mechanical and 15 bioprostheses). Seven children died during surgery. The operative mortality was higher before 2 years of age (4/7) than in older children (3/18). It was also higher when valve replacement was performed at second intention (5/14) than when decided on from the outset (2/11). With an average follow-up over 3 years, 9 of the 18 survivors are considered to be good surgical results. Of the other 9, 3 are considered to be moderate results (2 associated subaortic stenoses), 3 present indications of reoperation for calcification of the bioprosthesis, and 3 died in the late follow-up period. This series suggests that plastic surgery of congenital malformations of the atrioventricular valves should continue to be developed.
A series of 100 patients with complex forms of transposition of the great arteries (TGA) were operated upon over a 10 year period. Group 1 consisted of 13 TGA with pulmonary stenosis (PS), usually treated by an atrial baffle and direct repair of the stenosis. There was no operative or late mortality in this group and the long-term results were generally good (10/13). Group II comprised 29 TGA with ventricular septal defect (VSD) and PS. Seven Rastelli procedures gave 4 good results. Twenty two operations, associating atrial baffle, repair of VSD and PS (17 direct procedures, 5 left ventricle-pulmonary artery tube) were associated with a high mortality (5 operative and 4 late deaths) and 10 good long-term results. Group III comprised 58 TGA with VSD. Up to 1977 (n = 33) treatment consisted of atrial baffle + repair of VSD +/- removal of previous banding. Mortality was high (11 operative and 8 late deaths) with only 8 good long-term results. Since 1977, these patients have been treated by complete anatomical repair, the operative mortality of which is higher in the period under study (9/23) but the long-term results are much better. The surgical indications in our Department are based on the analysis of these results. In Group I only very significant PS is treated, either by left ventricle-pulmonary artery tube or direct repair depending on the form of the stenosis. In Group II, where the results based on atrial baffle are poor, a Rastelli procedure is preferred and especially its variants which avoid the use of prosthetic materials on the pulmonary trunk. In Group III, anatomical correction at the level of the great arteries is the routine procedure, the only point of discussion being the possibility of prior banding.
The results of palliative surgery are assessed in a retrospective study of 228 children operated between 1968 and 1977 for complex cyanotic congenital cardiac malformations, the longest follow-up period being 10 years. The surgery consisted in revascularising the lung by systemo-pulmonary anastomoses: Blalock-Taussing, Potts, Waterston, cavo-pulmonary shunts or, on the contrary, of avoiding pulmonary hypertension by limiting pulmonary flow with banding. In addition to these two techniques, in some cases atrial septal defects were created to improve mixing of the two circulations; equalise the pressures between the two atria and relieve the left ventricle (Blalock-Hanlon procedure) or during catheterisation (Rashkind manoeuvre). The results of pulmonary revascularisation by systemo-pulmonary anastomoses were generally satisfactory with respect to the complexity of the malformations. There were 56% good results, 82 out of 146 cases. It often provided the time for curative surgery to be performed under better conditions for the children. The results were worse in children with pulmonary hypertension, with only 33% of good results, 25 out of 82 children, and there were many complications of banding which additional problems at the time of definitive repair. Despite the tendency to widen the indications of so-called curative surgery even in very small infants, palliative surgery retains a role in complex cardiac malformations either by allowing the children to reach an optimal age for repair or by being the only possible therapeutic attitude.
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New techniques of correction of complex congenital anomalies, avoiding the use of prosthetic conduits, are presented. In transposition of the great arteries (TGA) with ventricular septal defect (VSD) and pulmonary stenosis, the technique comprised the resection of infundibular septum, the suturing of an intraventricular baffle directing blood from the left ventricle to the aorta, and the reconstruction of the pulmonary outflow tract by direct implantation of the posterior rim of the pulmonary arterial trunk on the right ventricle, completed by an anterior patch. In most cases, the pulmonary bifurcation was translated anterior to the ascending aorta. This technique was feasible even in infants and in patients with a small VSD. Thirteen patients, from 3 months to 8 years of age, were treated by this technique, with four deaths, one residual VSD (reoperated), and eight good results (4 to 16 months). A similar repair was used in three cases of double-outlet right ventricle (DORV) with subpulmonic VSD and pulmonary stenosis or pulmonary artery banding, with two operative deaths and one good result. The same technique of pulmonary outflow tract reconstruction was used in four cases of truncus arteriosus. Two deaths were attributed to severe pulmonary regurgitation, a complication which should be prevented in future cases by a reliable method of inserting a valve in the pulmonary outflow tract. In pulmonary atresia with VSD and absent pulmonary trunk, the continuity between the right ventricle and the pulmonary branches was established via an arterial tube resected from the ascending aorta. This technique was successfully used in one child with extremely small pulmonary branches. These preliminary results led us to conclude that many complex congenital cardiac anomalies can be effectively treated without a prosthetic conduit.
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142 infants under 6 months of age were operated on for coarctation of the aorta from 1972 to 1978. Coarctation was isolated in 46 (32.4%) and associated with another heart defect in 96. Surgery was decided upon because of congestive heart failure sometimes associated with arterial hypertension refractory to medical treatment: 9 patients died during surgery. 89 underwent Crafoord's operation and in 44 ligature of the subclavian artery or aortoplasty using the subclavia were carried out. Early postoperative death rate was 30.9% and 13 patients (13.2%) died later. Recoarctations occurred in 25 patients (27%); more than half of these patients were less than 1 month-old at the time of surgery. Recoarctation was more frequent (46.4%) in cases with than in cases without (18.7%) aortic hypoplasia and it was more severe (5 out of 7 deaths) when there was an associated cerclage resulting in a double obstacle to right and left ventricles ejection.
Iatrogenic arteriovenous fistulas in infants are usually due to an arterial puncture performed in the neonatal period. On the occasion of 5 cases who were operated on, the peculiar features of these fistulas are established. Surgery consists of endo-aneurysmorrhaphy.
Cor triatriatum is a rare heart defect that may be surgically repaired, with good results when preoperative diagnosis is correct. Ultrasonography, angiography and catheterism reduce preoperative diagnostic errors such as abnormal pulmonary venous return. The authors report their experience with 7 cases, and discuss diagnostic problems and causes of errors.
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The authors report a case of recurrent cerebral embolism in a 43-year-old patient with a familial form of Rendu-Osler-Weber disease and review their experience of 19 cases of pulmonary arteriovenous fistula. Neurological complications were rare but considerably aggravated the disease. They were due to a significant right/left shunt but remained unpredictable, being unrelated to peripheral arterial saturation. The pathophysiological mechanisms and therapeutic possibilities are discussed.